AHA/ACC: Shared decision-making key in diagnosis, treatment of hypertrophic cardiomyopathy
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The role of shared decision-making, prevention strategies for sudden cardiac death and recommendations for exercise are among the key points of an updated guidance for the diagnosis and treatment of hypertrophic cardiomyopathy.
The joint American Heart Association/American College of Cardiology update, published in Circulation and the Journal of the American College of Cardiology, added clinical data and new expert recommendations to the previous 2011 guideline publication.
“Shared decision-making, a dialogue between patients and their care team that includes full disclosure of all testing and treatment options, discussion of the risks and benefits of those options and, importantly, engagement of the patient to express their own goals, is particularly relevant in the management of conditions such as hypertrophic cardiomyopathy,” Steve R. Ommen, MD, FACC, FAHA, professor of medicine and consultant in the department of cardiovascular medicine at the Mayo Clinic College of Medicine, and chair of the writing committee for the guideline, said in a press release. “This updated guideline places emphasis on including the patient in the decision-making process rather than simply providing dogmatic lists of do’s and don’ts.”
One of the main takeaways was the updated guidance on shared decision-making between the patients and their care team. Points of shared decision-making include discussion on testing and treatment options for hypertrophic cardiomyopathy, risks and benefits of those options and expression of the patient’s own goals.
The guidance also noted the potential for referral to multidisciplinary hypertrophic cardiomyopathy centers with graduated levels of expertise for the optimization of care.
Ommen told Healio that “family screening recommendations were broadened so that more patients may be diagnosed earlier in the course of their disease.”
According to the update, patients should be made aware of the potential for genetic transmission of hypertrophic cardiomyopathy and screening first-degree family members can begin at any age. Reported pathogenicity should be reconfirmed every 2 to 3 years.
Risk for sudden cardiac death
According to the statement, optimal care in hypertrophic cardiomyopathy includes cardiac imaging to confirm diagnosis, characterize the pathophysiology for the individual and identify risk markers that may inform decisions regarding interventions for the prevention of left ventricular outflow tract obstruction and sudden cardiac death.
“Sudden cardiac death risk stratification evolved and now the toolset, independently produced by the ACC and AHA, allows use of risk factor-based recommendations and 5-year risk prediction to help patients understand the magnitude of their risk as they contemplate whether to pursue [implantable cardioverter defibrillator] placement,” Ommen said in an interview, “However, sudden cardiac death risk assessment continues to evolve and the current prediction tool does not account for several of the newer risk markers.”
Individual risk for sudden cardiac death changed as new markers that included apical aneurysm, decreased LV systolic function and extensive gadolinium enhancement emerged. Communication regarding the presence of risk markers and their magnitude of risk for sudden cardiac death may be key, the authors wrote.
Moreover, risk factors for sudden cardiac death among children with hypertrophic cardiomyopathy are different compared with those observed in adults, vary with age and must account for different body sizes. Due to the complexity of placing ICDs in younger patients, with anticipated growth and elevated risk for device complications, the threshold for implantation often differs compared with adults, according to the guidance, and these differences are best addressed at primary or comprehensive hypertrophic cardiomyopathy centers.
Ommen added that within the 2020 update, “the roles of surgical myectomy and septal ablation were more specifically defined.”
Septal reduction therapies continue to show good safety and efficacy, according to the statement. Because of this, earlier intervention may be possible among those with drug-refractory or severe outflow tract obstruction causing signs of cardiac decompensation, according to the update.
Symptoms of HF in hypertrophic cardiomyopathy, without LV outflow tract obstruction, can be treated similarly to other patients with HF symptoms. According to the updated guidance, this extended to the use of advanced treatment options such as cardiac resynchronization therapy, LV assist devices or transplantation. Moreover, an ejection fraction of less than 50% in hypertrophic cardiomyopathy is indicative of impaired systolic function and identifies individuals at increased risk for sudden cardiac death.
Exercise in hypertrophic cardiomyopathy
The updated guidance also addressed the positive health effects of exercise; how these benefits may extend to patients with hypertrophic cardiomyopathy; and that moderate-intensity exercise has not been associated with risk for ventricular arrhythmia events. A move to intense physical activity should be dependent on shared discussion between the patient and their hypertrophic cardiomyopathy care team.
Ommen told Healio that “research into the safety of higher intensity exercise or athletic participation needs to continue. [Also] the ‘place-in-therapy’ for mavacamten (MyoKardia), considering the excellent outcomes for current therapies (eg, myectomy performed at expert centers), is not yet clear.”
For more information:
Steve R. Ommen, MD, FACC, FAHA, can be reached at ommen.steve@mayo.edu.
Reference:
Perspective
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John D. Symanski, MD, FACC, FASE
This much-anticipated guideline incorporates a wealth of clinical data and expert recommendations that have accrued since the previous guideline publication in 2011.
A key recommendation in the 2020 update is the provision for a shared decision-making process between clinician and patient that includes the full disclosure of testing and treatment options. Patients with hypertrophic cardiomyopathy have an abundance of access to important information about their condition through organizations such as the Hypertrophic Cardiomyopathy Association (HCMA) as well as through social media. Such knowledge empowers patient engagement in the hypertrophic cardiomyopathy shared decision-making dialogue.
While the guidelines provide clinicians with a foundation for the evaluation and management of hypertrophic cardiomyopathy, it is important to recognize that this an incredibly complex and nuanced condition. The value of referral to dedicated hypertrophic cardiomyopathy centers for integrated care, including advanced imaging with MRI and cardiac CT, genetic testing and counseling, operators experienced with septal reduction procedures, atrial fibrillation management and advanced HF therapies, is stressed.
Another area of emphasis in the guideline update is the encouragement of a healthy lifestyle and allowance of moderate intensity recreational activity for the majority of patients with hypertrophic cardiomyopathy. Traditionally, exercise has been discouraged for these patients, depriving them of the recognized psychologic and physiologic benefits. Lack of physical activity contributes to a heightened incidence of obesity, diabetes and obstructive sleep apnea — conditions that lead to further worsening hypertrophic cardiomyopathy symptoms. Mounting evidence affirms that recreation exercise is safe and is not associated with increased risk for ventricular arrhythmia.
Finally, the ongoing assessment of risk for sudden cardiac death remains one of the most important and challenging aspects of hypertrophic cardiomyopathy care. Risk assessment should be conducted during the course of an initial comprehensive evaluation and revisited at 1-to-2-year intervals thereafter. In addition to the traditional hypertrophic cardiomyopathy sudden death risk factors (family history of hypertrophic cardiomyopathy-related sudden death, severe hypertrophy, recent syncope and repetitive nonsustained ventricular tachycardia), recognition of novel risk factors (apical aneurysm, scar burden assessed by MRI late gadolinium enhancement and LV ejection fraction < 50%) will hopefully improve the selection of appropriate candidates for primary prevention automated ICD therapy.
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